Academic Journal
Zebrafish disease model of human RNASET2-deficient cystic leukoencephalopathy displays abnormalities in early microglia
| العنوان: | Zebrafish disease model of human RNASET2-deficient cystic leukoencephalopathy displays abnormalities in early microglia |
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| المؤلفون: | Thomas Weber, Lars Schlotawa, Roland Dosch, Noémie Hamilton, Jens Kaiser, Stina Schiller, Britta Wenske, Jutta Gärtner, Marco Henneke |
| المصدر: | Biology Open, Vol 9, Iss 5 (2020) |
| بيانات النشر: | The Company of Biologists |
| سنة النشر: | 2020 |
| المجموعة: | Directory of Open Access Journals: DOAJ Articles |
| مصطلحات موضوعية: | cystic leukoencephalopathy, innate immune system, zebrafish, lysosomes, rnaset2, Science, Biology (General), QH301-705.5 |
| الوصف: | Human infantile-onset RNASET2-deficient cystic leukoencephalopathy is a Mendelian mimic of in utero cytomegalovirus brain infection with prenatally developing inflammatory brain lesions. We used an RNASET2-deficient zebrafish model to elucidate the underlying disease mechanisms. Mutant and wild-type zebrafish larvae brain development between 2 and 5 days post fertilization (dpf) was examined by confocal live imaging in fluorescent reporter lines of the major types of brain cells. In contrast to wild-type brains, RNASET2-deficient larvae displayed increased numbers of microglia with altered morphology, often containing inclusions of neurons. Furthermore, lysosomes within distinct populations of the myeloid cell lineage including microglia showed increased lysosomal staining. Neurons and oligodendrocyte precursor cells remained unaffected. This study provides a first look into the prenatal onset pathomechanisms of human RNASET2-deficient leukoencephalopathy, linking this inborn lysosomal disease to the innate immune system and other immune-related childhood encephalopathies like Aicardi-Goutières syndrome (AGS). |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 2046-6390 |
| Relation: | http://bio.biologists.org/content/9/5/bio049239; https://doaj.org/toc/2046-6390; https://doaj.org/article/94cf29131fb240afb6618a775d7bdd8e |
| DOI: | 10.1242/bio.049239 |
| الاتاحة: | https://doi.org/10.1242/bio.049239 https://doaj.org/article/94cf29131fb240afb6618a775d7bdd8e |
| رقم الانضمام: | edsbas.6BD16FA7 |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 20466390 |
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| DOI: | 10.1242/bio.049239 |